Literature DB >> 22453074

Myelodysplasia followed by Good's Syndrome: a unique manifestation associated with thymoma.

Ling-Ping Chen1, Jui-Shan Tsai, Wei-Ming Lai, Ling-Jung Yen, Ming-Sun Yu, Shyh-Jer Lin.   

Abstract

Good's syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good's syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good's syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good's syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.
Copyright © 2012. Published by Elsevier B.V.

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Year:  2012        PMID: 22453074     DOI: 10.1016/j.kjms.2011.10.012

Source DB:  PubMed          Journal:  Kaohsiung J Med Sci        ISSN: 1607-551X            Impact factor:   2.744


  3 in total

1.  Thymoma associated with hypogammaglobulinaemia and pure red cell aplasia.

Authors:  Juan Briones; Mirentxu Iruretagoyena; Héctor Galindo; Claudia Ortega; Pablo Zoroquiain; José Valbuena; Francisco Acevedo; Mauricio Ocqueteau; Cesar Sánchez
Journal:  Ecancermedicalscience       Date:  2013-10-17

Review 2.  Characteristics of Good's Syndrome in China: A Systematic Review.

Authors:  Jin-Pei Dong; Wen Gao; Gui-Gen Teng; Yu Tian; Hua-Hong Wang
Journal:  Chin Med J (Engl)       Date:  2017-07-05       Impact factor: 2.628

3.  Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia).

Authors:  M Zaman; A Huissoon; M Buckland; S Patel; H Alachkar; J D Edgar; M Thomas; G Arumugakani; H Baxendale; S Burns; A P Williams; S Jolles; R Herriot; R B Sargur; P D Arkwright
Journal:  Clin Exp Immunol       Date:  2018-10-21       Impact factor: 4.330

  3 in total

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